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Biomedical subjects

R R Heffner

Publications and source records attributed to R R Heffner.

50 records · Page 3Linked to original sources

Brain scanning in oligodendrogliomas: a detailed neuropathology-scan correlation of 34 histologically verified cases.

Thirty-six brain scans and biopsies from 34 patients with histologically verified oligodendrogliomas were evaluated. Twenty-nine of the 36 scans were positive (80.6%) but the abnormal uptake produced by these neoplasms had no distinguishing features. The levels of endothelial proliferation-vascularity, necrosis, and mitoses were significantly different between the positive and negative scans. In the oligodendrogliomas, the relationship between histologic malignancy, detectability on scan, and prognosis remains unresolved.

Adolescent↗

Syndrome of absent abdominal muscles: two cases with microcephaly, polymicrogyria, and cerebellar malformations.

Two unique cases of the syndrome of absent abdominal muscles with central nervous system involvement are presented. Microcephaly, polymicrogyria, and cerebellar heterotopiae were present in both. In case 1 there was also absence of the corpus callosum and agenesis of the cerebellar vermis. In case 2 a count of anterior horn cells in the spinal cord showed a reduction of approximately 50% in the lower thoracic region. The pertinent literature is briefly discussed. The findings in the nervous system suggest that the syndrome is the result of defective embryogenesis during the first trimester.

Abdominal Muscles↗

Delayed functional disability in dystrophic chickens receiving chemotherapy.

Line 413 early-onset, genetically homozygous dystrophic chickens were given twice-daily intraperitoneal injections of the antiserotoninergic drug cinanserin, alone or in combination with methysergide. Other trials consisted of penicillamine treatment in combination with either methysergide or cyproheptadine. Chemotherapy significantly prolonged the righting ability of treated dystrophic chickens, as measured by a periodic standardized flip-test procedure. Plasma creatine kinase activities were not affected by any of the various drug treatments. However, the blood serotonin levels of the dystrophic chickens (grand mean 1.47 microgram serotonin/ml blood) were found to be significantly higher (p less than 0.001) than those in the corresponding normal chickens (0.99 microgram/ml). This finding may partially account for the antiserotoninergic drug enhancement in righting ability that was demonstrated in the drug-treated dystrophic chickens.

Animals↗

Parenteral branched-chain amino acid treatment and avian dystrophy.

Genetically homozygous line 413 dystrophic chickens were given twice-daily intraperitoneal injections of solutions containing branched-chain amino acids (BCCA-leucine, valine, isoleucine) either alone or in combination; and their alpha-ketoacid analogs (alpha-ketoisocaproic and alpha-ketoisovaleric acids). Another trial consisted of an amino acid mixture containing BCAA. Amino acid supplementation in each case significantly prolonged righting ability measured regularly by a standardized flip-test procedure. Enhanced functional ability was not generally accompanied by a decrease in plasma creatine kinase activity. However, a measurable increase in the affected pectoralis major muscle mass and protein content (female chickens in particular) was found with BCAA therapy. Moreover, the increase in muscle bulk was attended in some cases by a reduction in the relative number of degenerating fibers quantitated microscopically. Contrariwise, the amino acid mixture caused a reduction in pectoralis muscle mass. It is concluded that parenteral BCAA therapy offers limited benefit in retarding dystrophic symptoms in the chickens.

Amino Acids↗

Electron microscopy of disorders of skeletal muscle.

Some of the common pathologic changes in the muscle fiber as viewed with the electron microscope are considered. These include myofilament and Z band abnormalities, alterations of the sarcotubular system, mitochondrial aberrations and glycogen accumulations. Correlations with light microscopic findings are mentioned when pertinent. The diagnostic significance of the various ultrastructural changes is discussed.

Glycogen↗

Adult central core disease. Clinical, histologic and genetic aspects: case report and review of the literature.

Central core disease (CCD) is mainly a disease of infancy and childhood and represents a member of a group of muscular disorders known as "congenital, benign (non-progressive) myopathies". It is an uncommon disease of infancy and early childhood, and presentation is rare in adulthood. The disease is mainly familial with an autosomal-dominant pattern of inheritance, yet sporadic cases can occur. The diagnosis is based on a muscle biopsy, which documents unique morphological abnormalities of focal loss of oxidative enzyme in type I muscular fibers. The basis for this loss of such activities is represented by a near-total absence of mitochondria and sarcoplasmic reticulum in the cores. We describe a 58-year-old man diagnosed with CCD, who is one of the oldest individuals reported with CCD diagnosed by a muscle biopsy. The clinical, pathological and genetic features of this rare entity are discussed herein.

Adenosine Triphosphatases↗